{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T18:59:13Z","timestamp":1777489153984,"version":"3.51.4"},"reference-count":16,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2010,5,13]],"date-time":"2010-05-13T00:00:00Z","timestamp":1273708800000},"content-version":"vor","delay-in-days":132,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Laboratory Analysis"],"published-print":{"date-parts":[[2010,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:italic>Background<\/jats:italic>: The fact that mucopolysaccharidoses (MPSes) are now treatable, and that the earlier treatment is initiated the better, is an indication for neonatal screening. The most efficient approach seems likely to be a multi\u2010tier procedure in which screening for urinary glycosaminoglycan (GAG) is followed by enzyme determinations in heelprick blood of newborns screening positive. Hitherto the method of choice for the determination of GAG has been the measurement of absorbance by a complex of GAG and 1,9\u2010dimethylmethylene blue (DMB). <jats:italic>Method<\/jats:italic>: We evaluated a DMB method in which absorbance by DMB is measured following its addition to the eluate obtained from paper\u2010borne newborn urine samples and is normalized relative to urinary creatinine. Calibration is performed with chondroitin\u20106\u2010sulfate (Ch\u20106\u2010S). <jats:italic>Results<\/jats:italic>: The limits of detection and quantification of GAG were 1.98 and 5.94\u2009mg\/dl, respectively. The within\u2010run coefficients of variation (CVs) of the GAG\/creatinine ratio for 25, 31, and 70\u2009mg\/dl solutions of Ch\u20106\u2010S in urine were 21.8, 16.4, and 10.5%, respectively, and the corresponding between\u2010run CVs were 25.0, 13.5, and 10.1%. Recovery from the urine spiked with 31\u2009mg Ch\u20106\u2010S\/dl was 94.8%. Accuracy was also acceptable for all other GAGs except hyaluronic acid. For neonatal screening, the diagnostic threshold was tentatively established as 800\u2009mg GAG\/g creatinine, the 95th centile of samples from 903 infants aged 3\u201328 days, but the value of the GAG\/creatinine ratio was negatively correlated with age. Application of the new method to samples from older individuals with and without MPS achieved 100% sensitivity and specificity when used with an age\u2010dependent threshold taken from the literature on the original DMB method. <jats:italic>Conclusion<\/jats:italic>: If used in the first tier of a multi\u2010tier screening protocol, the proposed method would allow the detection of abnormal levels of all GAGs except hyaluronic acid. J. Clin. Lab. Anal. 24:149\u2013153, 2010. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jcla.20375","type":"journal-article","created":{"date-parts":[[2010,5,14]],"date-time":"2010-05-14T14:49:20Z","timestamp":1273848560000},"page":"149-153","source":"Crossref","is-referenced-by-count":21,"title":["Neonatal screening for mucopolysaccharidoses by determination of glycosaminoglycans in the eluate of urine\u2010impregnated paper: preliminary results of an improved DMB\u2010based procedure"],"prefix":"10.1002","volume":"24","author":[{"given":"J.R.","family":"Alonso\u2010Fern\u00e1ndez","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Fidalgo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Col\u00f3n","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2010,5,13]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/5.6.603"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0009-9120(87)80088-7"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1973.tb01152.x"},{"key":"e_1_2_1_5_2","doi-asserted-by":"crossref","first-page":"2074","DOI":"10.1093\/clinchem\/35.10.2074","article-title":"Diagnostic test for mucopolysaccharides. 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Rapid quantification of glycosaminoglycan in urine samples collected on paper matrix","volume":"35","author":"Whitley CB","year":"1989","journal-title":"Clin Chem"},{"key":"e_1_2_1_6_2","first-page":"21","article-title":"A colorimetric method for the determination of sulphated glycosaminoglycans","volume":"11","author":"Humbel R","year":"1974","journal-title":"Rev Roum Biochem"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1006\/mgme.2001.3271"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2008.00652.x"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(92)90134-C"},{"key":"e_1_2_1_10_2","first-page":"481","volume-title":"Advances in Neonatal Screening","author":"Fraga JM","year":"1987"},{"key":"e_1_2_1_11_2","volume-title":"Phenylketonuria and allied metabolic diseases","author":"Woolf LI","year":"1967"},{"key":"e_1_2_1_12_2","first-page":"278","article-title":"Determinaci\u00f3n de glucosaminoglicanos en orina por un procedimiento espectrom\u00e9trico. Valores de referencia para una poblaci\u00f3n pedi\u00e1trica","volume":"18","author":"Colom\u00e9 Mallolas C","year":"1999","journal-title":"Qu\u00edmica Cl\u00ednica"},{"key":"e_1_2_1_13_2","first-page":"A 209","article-title":"A pediatric reference range for urinary glycosaminoglycans using the VITROS 5, 1FS chemistry system","volume":"53","author":"Molinelli AR","year":"2007","journal-title":"Clin Chem"},{"key":"e_1_2_1_14_2","first-page":"36","article-title":"Comit\u00e9 Cient\u00edfico. Comisi\u00f3n de Errores Metab\u00f3licos Cong\u00e9nitos. Document B, phase 3, version I. Prepared by I. Igueleor Guturbai, M. Espada S\u00e1enz\u2010Torre, E. Dulin I\u00f1iguez, F. Chamorro Ure\u00f1a","volume":"25","author":"Sociedad Espa\u00f1ola de Bioqu\u00edmica Cl\u00ednica y Patolog\u00eda Molecular (SEQC)","year":"2006","journal-title":"Qu\u00edmica Cl\u00ednica"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1136\/fn.82.1.F77b"},{"key":"e_1_2_1_16_2","unstructured":"Gu\u00edas de Sa\u00fade Publica.Serie II: Secci\u00f3n E. Endocrinas e Metab\u00f3licas. Informe 3. Actualizaci\u00f3n do Programa Galego para a Detecci\u00f3n de Enfermidades Endocrinas e Metab\u00f3licas en Per\u00edodo Neonatal. Resultados 1995\u20132005. Novembro 2006. Xunta de Galicia. Conseller\u00eda de Sanidade. Direcci\u00f3n Xeral de Sa\u00fade P\u00fablica. Servicio de Programas Poblacionais de Cribado. Dep\u00f3sito Legal: C\u20133031\u20132006."},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1093\/clinchem\/47.12.2098"}],"container-title":["Journal of Clinical Laboratory Analysis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fjcla.20375","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jcla.20375","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,21]],"date-time":"2023-11-21T15:59:58Z","timestamp":1700582398000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jcla.20375"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,1]]},"references-count":16,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2010,1]]}},"alternative-id":["10.1002\/jcla.20375"],"URL":"https:\/\/doi.org\/10.1002\/jcla.20375","archive":["Portico"],"relation":{},"ISSN":["0887-8013","1098-2825"],"issn-type":[{"value":"0887-8013","type":"print"},{"value":"1098-2825","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,1]]}}}